Linear IgA Dermatosis Workup

  • Author: Peter A Klein, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 7, 2012
 

Laboratory Studies

Direct immunofluorescence study of both perilesional skin and healthy skin typically shows linear deposition of IgA at the BMZ. Linear deposition of C3 may also be seen. Direct immunofluorescence study of salt-split skin reveals IgA deposition on either the dermal side (blister floor) or the epidermal side (blister roof). Some patients demonstrate both linear IgA deposition and immunoglobulin G (IgG) deposition at the BMZ. Immunoglobulin M (IgM) deposition has rarely been reported.

Serum should be obtained for indirect immunofluorescence studies. Approximately 50% of patients with linear IgA dermatosis have detectable circulating antibody that binds to the BMZ. Sensitivity is greater for immunofluorescence studies performed on salt-split healthy human skin. Circulating antibody titers are typically low (1:10 to 1:20). When present, linear deposition of antibody is observed at the BMZ or at the blister roof or floor in salt-split skin. Children with linear IgA dermatosis may demonstrate circulating anti-BMZ antibodies more frequently than adults.

In patients with atypical presentations, additional testing, including bacterial culture and Gram stain of blister fluid to rule out bullous impetigo and Tzanck smear to rule out herpes virus infection, may be helpful.

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Histologic Findings

Early urticarial papules or plaques reveal neutrophils aligned along the BMZ accompanied by vacuolar change. Neutrophilic microabscesses may be seen in dermal papillae (see the image below).

Neutrophilic microabscesses in linear immunoglobulNeutrophilic microabscesses in linear immunoglobulin A (IgA) dermatosis.

Fully developed lesions reveal subepidermal blistering with a predominantly polymorphonuclear infiltrate, although mononuclear cells and eosinophils may be present. Obtaining a frozen section of a blister roof may be helpful in some patients to rule out full-thickness epidermal necrosis as seen in toxic epidermal necrolysis.

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Contributor Information and Disclosures
Author

Peter A Klein, MD  Residency Program Director, Department of Dermatology, University Hospital, State University of New York at Stony Brook

Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Celgene Honoraria Safety Monitoring Committee

Specialty Editor Board

Russell Hall, MD  J Lamar Callaway Professor And Chair, Department of Dermatology, Duke University Medical Center, Duke University School of Medicine

Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, American Society for Clinical Investigation, and Society for Investigative Dermatology

Disclosure: Genetech Grant/research funds Principle Investigator; Centecor Grant/research funds Principle Investigator; Vernallis Honoraria Consulting

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

References

  1. Zone JJ, Taylor TB, Meyer LJ, Petersen MJ. The 97 kDa linear IgA bullous disease antigen is identical to a portion of the extracellular domain of the 180 kDa bullous pemphigoid antigen, BPAg2. J Invest Dermatol. Mar 1998;110(3):207-10. [Medline].

  2. Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group. Arch Dermatol. Jan 1995;131(1):48-52. [Medline].

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  4. Wojnarowska F, Marsden RA, Bhogal B, Black MM. Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap. J Am Acad Dermatol. Nov 1988;19(5 Pt 1):792-805. [Medline].

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  11. Yhim HY, Kwon DH, Lee NR, Song EK, Yim CY, Kwak JY. Linear IgA bullous dermatosis following autologous PBSC transplantation in a patient with non-Hodgkin's lymphoma. Bone Marrow Transplant. Apr 12 2010;[Medline].

  12. Godfrey K, Wojnarowska F, Leonard J. Linear IgA disease of adults: association with lymphoproliferative malignancy and possible role of other triggering factors. Br J Dermatol. Oct 1990;123(4):447-52. [Medline].

  13. Krejci-Manwaring J, West DA, Tonkovic-Capin V. If at first you don't succeed: a difficult case of Linear IgA. Dermatol Online J. Sep 15 2009;15(9):16. [Medline].

  14. Jablonska S, Chorzelski TP, Rosinska D, Maciejowska E. Linear IgA bullous dermatosis of childhood (chronic bullous dermatosis of childhood). Clin Dermatol. Jul-Sep 1991;9(3):393-401. [Medline].

  15. Klein PA, Callen JP. Drug-induced linear IgA bullous dermatosis after vancomycin discontinuance in a patient with renal insufficiency. J Am Acad Dermatol. Feb 2000;42(2 Pt 2):316-23. [Medline].

 
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Annular lesions demonstrating the string of beads sign.
Bullous lesions on the genital area in a child with linear IgA dermatosis.
Persons with linear immunoglobulin A (IgA) dermatosis may present with prominent ocular signs and symptoms.
Neutrophilic microabscesses in linear immunoglobulin A (IgA) dermatosis.
 
 
 
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